Segmental spinal dysgenesis: insights from three consecutive cases and a review of the literature.

OBJECTIVE: Segmental spinal dysgenesis (SSD) is a rare congenital spinal abnormality affecting the thoracic and lumbar region of the spine, as well as the spinal cord of neonates and infants. The purpose of the study was to analyze our institution's surgical case series to provide insights into our best practices to contribute to SSD management principles, while conducting a comprehensive literature review. METHODS: Following institutional review board approval, a retrospective review of SSD surgical cases was examined to observe clinical findings, radiographic findings, management, surgical intervention, and outcomes. Keywords in the comprehensive literature review included SSD, congenital spinal dysgenesis, congenital spinal stenosis, spinal aplasia, and surgery. RESULTS: Three cases underwent successful surgical management with either improvement or maintenance of neurological baseline. Patients were diagnosed at an average age of 2.7 months, while surgical intervention averaged at 40.3 months with fecal incontinence, neurogenic bladders, spinal cord compression, clubfoot, and concerns for worsening spinal deformity as surgical indicators. The average time for follow-up was 33.7 months and no complications were reported. CONCLUSIONS: Operative management for SSD is a clinically complex decision that requires multidisciplinary input and care. Patients should be observed at neurological baseline and receive intervention at the appropriate time to allow sufficient growth for functioning without permitting drastic disease progression. Consideration of patient size and spinal instrumentation are significant towards surgical success.

Technical case report: intractable focal seizures related to bifrontal transmantle heterotopia subserving peculiar homotopic motor distribution treated by responsive neurostimulation therapy.

NeuroPace responsive neurostimulation (RNS®) therapy was used in a case of intractable focal epilepsy with bifrontal transmantle heterotopia subserving peculiar homotopic motor distribution in a 16-year-old, right-handed male with intractable seizures. Brain MRI demonstrated bifrontal transmantle heterotopia extending from the central sulcus to subjacent lateral ventricles along with polymicrogyria along the overlying cortex suspected to be the motor cortex. Functional MRI demonstrated homotopic distribution of finger and foot motor function (deeper) within the polymicrogyria. Invasive intracranial monitoring with depth electrodes and extraoperative brain mapping revealed eloquent cortical tissue which corresponded to the right leg and right shoulder motor function.

Virtual reality-based 3-dimensional localization of stereotactic EEG (SEEG) depth electrodes and related brain anatomy in pediatric epilepsy surgery.

INTRODUCTION: The increasing use of stereoelectroencephalography (SEEG) in the USA and the need for three-dimensional (3D) appreciation of complex spatial relationships between implanted stereotactic EEG depth electrodes and surrounding brain and cerebral vasculature are a challenge to clinicians who are used to two-dimensional (2D) appreciation of cortical anatomy having been traditionally trained on 2D radiologic imaging. Virtual reality and its 3D renderings have grown increasingly common in the multifaceted practice of neurosurgery. However, there exists a paucity in the literature regarding this emerging technology in its utilization of epilepsy surgery. METHODS: An IRB-approved, single-center retrospective study identifying all SEEG pediatric patients in which virtual reality was applied was observed. RESULTS: Of the 46 patients identified who underwent an SEEG procedure, 43.5% (20/46) had a 3D rendering (3DR) of their SEEG depth electrodes. All 3DRs were used during patient-family education and discussion among the Epilepsy multidisciplinary team meetings, while 35% (7/20) were used during neuronavigation in surgery. Three successful representative cases of its application were presented. DISCUSSION: Our institution's experience regarding virtual reality in the 3D representation of SEEG depth electrodes and the application to pre-surgical planning, patient-family education, multidisciplinary communication, and intraoperative neuronavigation demonstrate its applicability in comprehensive epilepsy patient care.

Responsive neurostimulation for the treatment of medically refractory epilepsy in pediatric patients: strategies, outcomes, and technical considerations.

OBJECTIVE: Children with medically refractory partial-onset epilepsy arising from eloquent cortex present a therapeutic challenge, as many are not suitable for resective surgery. For these patients, responsive neurostimulation may prove to be a potential tool. Although responsive neurostimulation has demonstrated utility in adults, little has been discussed regarding its utility in the pediatric population. In this study, the authors present their institution's experience with responsive neurostimulation via the RNS System through a case series of 5 pediatric patients. METHODS: A single-center retrospective study of patients who underwent RNS System implantation at Children's National Hospital was performed. RESULTS: Five patients underwent RNS System implantation. The mean patient age at treatment was 16.8 years, and the average follow-up was 11.2 months. All patients were considered responders, with a seizure frequency reduction of 64.2% without adverse events. CONCLUSIONS: All 5 patients experienced medium-term improvements in seizure control after RNS System implantation with decreases in seizure frequency > 50% from baseline preoperative seizure frequency. The authors demonstrated two primary configurations of electrode placement: hippocampal or amygdala placement via an occipitotemporal trajectory, as well as infratemporal surface electrodes and surface electrodes on the primary motor cortex. No adverse events were experienced in this case series.

The Effect of Morbid Obesity on Subarachnoid Hemorrhage Prognosis in the United States.

OBJECTIVE: The association between obesity and nontraumatic subarachnoid hemorrhage (SAH) patient outcome is unclear. The aim of this study was to determine the impact of morbid obesity (body mass index ≥40 kg/m2) on nontraumatic SAH outcomes. METHODS: Using the Nationwide Inpatient Sample, we identified hospitalized, nontraumatic SAH patients who received their diagnoses from 2008 to 2013 and tested the effect of obesity on their mortality and clinical outcomes. Odds ratios were estimated with a mixed effects linear logistic model with adjustment for hospital clustering. All statistical testing was 2-sided, with a significance level of 5%. RESULTS: Out of 224,561 discharged patients with a diagnosis of nontraumatic SAH, 4714 (2.10%) were defined as morbidly obese. Patients with morbid obesity were younger (54.3 ± 0.44 vs. 59.5 ± 0.08 years; P < 0.001) and had longer length of stay (LOS) (13 ± 0.46 vs. 11.5 ± 0.06 days; P = 0.002). Morbid obesity was associated with significantly higher hospital costs (P < 0.001) and charges (P < 0.001). The risk of acute respiratory failure was higher in morbidly obese patients (odds ratio [OR] 1.49, 95% confidence interval [CI]: 1.3-1.71, P < 0.001). In a multivariate analysis of hospital mortality, obesity had a negative impact on mortality (OR 0.83, 95% CI: 0.74-0.92, P < 0.001). Overall, in-hospital mortality was associated with age, morbid obesity, LOS, clipping and coiling, and acute respiratory failure but not the symptomatic vasospasm. CONCLUSIONS: Morbid obesity is associated with increased LOS, hospital costs and charges and with acute respiratory failure. However, it is also associated with a decrease in hospital mortality.